Ying-Ren Chen, Chien-Hsien Lai, Liang-Yi Hung and Kung-Chao Chang
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of low malignant potential that occurs mostly in soft tissue. So far, only two cases of EHE involving the intestine have been recorded. Here, we describe a rare case of cecal EHE due to a subsequent metastasis from the primary liver tumor three years after initial diagnosis. A 74- year-old man had sudden onset of epigastralgia for 24 hours. The abdominal CT revealed an ileocecal mass with a small bowel obstruction, and extensive tumor involvement in the liver was noted. He received an emergent right hemicolectomy for relief of the ileus. Unfortunately, he expired four days later due to septic shock. The pathologic diagnosis of EHE prompted a molecular study for a WWTR1-CAMTA1 fusion. The Sanger sequencing results showed the fusion involved exon 4 of WWTR1 with exon 8 of CAMTA1. There is no standard treatment for hepatic EHE because of its rarity and variable clinical outcome. The decision on a treatment strategy should be individualized for each patient. Since the patient received supportive care only for the liver tumor, this case demonstrated a natural course of hepatic EHE with a survival of more than 3.3 years.
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